Wiskott-Aldrich 综合征的前沿和热点演进：2001 年至 2021 年的文献计量分析。

Frontier and hotspot evolution in Wiskott-Aldrich syndrome: A bibliometric analysis from 2001 to 2021.

机构信息

Guanganmen Hospital, China Academy of Chinese Medical Sciences, Beijing, China.

Graduate School of Beijing University of Chinese Medicine, Beijing, China.

出版信息

Medicine (Baltimore). 2022 Dec 16;101(50):e32347. doi: 10.1097/MD.0000000000032347.

DOI:10.1097/MD.0000000000032347

PMID:36550896

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9771241/

Abstract

BACKGROUND

Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder. Despite our enormous progress in the strategies used to diagnose, treat, and cure WAS, no bibliometric studies have been performed in this research field. This study explored the trends in WAS research through a bibliometric analysis evaluating relevant literature quantitatively and qualitatively.

METHODS

The literature concerning WAS from 2001 to 2021 was retrieved from the Science Citation Index Expanded (SCI-expanded) of the Web of Science Core Collection database. Acquired data were then visually analyzed using CiteSpace and VOSviewer.

RESULTS

2036 papers were included in the final analysis. The annual publication outputs reached its peak in 2013 but declined in recent years. The dominant position of the United States in WAS research was quite obvious. Harvard University (USA), University College London (UK), and Inserm (France) were the three most prolific institutions. Adrian J. Thrasher exerted significant publication impact and made the most notable contributions in the field of WAS. Blood was the most influential journal with the highest publication outputs, and nearly all the top 10 journals and co-cited journals belonged to Q1. Immune dysregulation, thrombocytopenia, syndrome protein deficiency, stem cell, mutation, and diagnosis were the keywords with the strongest citation burst.

CONCLUSION

From 2001 to 2021, the United States was a global leader in the WAS research. Collaboration between countries and institutions is expected to deepen and strengthen in the future. Research hotspots included pathogenesis, clinical manifestations, diagnosis, and therapy. Our results suggest a greater understanding of the mechanistic underpinnings of immune dysfunction in WAS patients, the application of targeted therapies for individual complications, and the development of curative approaches, which will remain research hotspots in the future.

摘要

背景

Wiskott-Aldrich 综合征（WAS）是一种罕见的 X 连锁原发性免疫缺陷病。尽管我们在诊断、治疗和治愈 WAS 的策略上取得了巨大的进展，但在这个研究领域还没有进行过文献计量学研究。本研究通过文献计量分析，对相关文献进行了定量和定性评估，探讨了 WAS 研究的趋势。

方法

从 Web of Science 核心合集数据库的科学引文索引扩展版（SCI-expanded）中检索了 2001 年至 2021 年有关 WAS 的文献。使用 CiteSpace 和 VOSviewer 对获取的数据进行了可视化分析。

结果

最终分析纳入了 2036 篇论文。年度出版物数量在 2013 年达到顶峰，但近年来有所下降。美国在 WAS 研究中的主导地位非常明显。哈佛大学（美国）、伦敦大学学院（英国）和 Inserm（法国）是最有影响力的三个机构。Adrian J. Thrasher 在 WAS 领域的发表影响显著，做出了最显著的贡献。Blood 是最有影响力的期刊，发表的论文数量最多，排名前 10 的期刊和被引频次最高的期刊几乎都属于 Q1。免疫失调、血小板减少症、综合征蛋白缺乏、干细胞、突变和诊断是引用量最强的关键词。

结论

从 2001 年到 2021 年，美国在 WAS 研究方面处于全球领先地位。未来，国家和机构之间的合作有望加深和加强。研究热点包括发病机制、临床表现、诊断和治疗。我们的研究结果表明，对 WAS 患者免疫功能障碍的机制基础有了更深入的了解，针对个别并发症的靶向治疗得到了应用，以及治疗方法的发展，这些将是未来的研究热点。